You may remember my article entitled The trouble with the NET (Part 1)which was a lighthearted but still serious discussion about the dangers of self-treatment on the internet. Linked to that blog was a very popular article written by the scientists at Cancer Research UK debunking some cancer myths which seem to regularly patrol the NET and social media.
Many well meaning people will send you articles they saw on the ‘NET’ about this and that treatment which claims to cure cancer. The vast majority of them will be unfounded, lacking in any proper and formal scientific research and there is normally a product or book for sale attached to the link. In some instances, these ‘miracle cures’ can actually be dangerous for some.
In a follow on article, Cancer Research UK warns of the risks in Alternative Therapies which is written in a similar vein. I pay great attention to what these guys say. I know from my association with their research capability, that they take an evidence based approach and do not publish these things lightly. Please note Alternative Therapy is not the same as Complementary Therapy, it means an alternative to conventional treatment.
One bonus and very interesting aspect of their article is that they discuss the Steve Jobs issue of initially opting for alternative treatment rather than conventional, excellently making the point that he did not have Pancreatic Cancer, rather he had a Neuroendocrine Tumour (NET). We all know this, but many newspapers, magazines, TV commentators and bloggers frequently get this wrong.
Those selling and promoting alternative treatments rarely discuss the risks, especially the biggest risk of all – missing that small window of opportunity to hit a cancer with the best conventional (and proven) treatment possible.
The link to the article is here – I strongly encourage you to have a read – click here.
There’s another article here where a study confirms people who opt for alternative therapy die sooner than those who don’t – read here.
You may also be interested in reading Part 3 of this series where I discuss the FDA clampdown on bogus claims made by those sharing ‘miracle cures’
Be careful out there – it’s dangerous.
You may also like my Neuroendocrine Cancer myths articles – click here
One of the great things about learning is that it never ends 🙂 I came across this piece of information about how chemotherapy was invented. I had no idea. It began as a deadly cloud but it eventually ended up as a silver lining for certain cancer patients. It all began with the development of mustard gas and I’m sure we’ve all seen the awful pictures of solders leading each other from the battlefield having been affected by this ‘deadly cloud‘. Let’s hope we never have to witness that again. This weapon was first used 100 years ago this week (note: blog published in Apr 2015) but out of the horror came a ‘silver lining‘ – the idea behind what is now called chemotherapy.
However, the development didn’t really begin until the second world war when two doctors from Yale University (Louis Goodman and Alfred Gilman), conducted animal and then human trials. Then in 1948, UK scientist Professor Alexander Haddow published a ground breaking piece of research in the journal Nature, showing exactly which bits of the nitrogen mustard molecule were needed to kill cancer cells. Perhaps more importantly, he also found out how to make the chemical less toxic, but with more potent cancer-killing activity. So mustard gas went from the very real battleground of the WWI trenches into the frontline of cancer treatment where it still is today.
One of the unusual aspects of Neuroendocrine Cancer is that chemotherapy is not normally considered as a ‘standard’ treatment unlike many other cancers. The exception is high grade (Grade 3) where it is often a first and/or second line therapy. Poorly differentiated Neuroendocrine disease is normally labelled as Neuroendocrine Carcinoma (NEC) but worth pointing out there is now a Grade 3 well differentiated classification known as a ‘Grade 3 NET’ rather than Grade 3 NEC. Depending on Ki67 score, there could be different treatment options for Grade 3 NET and Grade 3 NEC. Read more in my article Staging and Grading.
The type of chemo or the combination of different treatments will also depend on the type and anatomical location of the High Grade tumour involved but may include (but not limited to) chemos such as Cisplatin, Etoposide, Carboplatin, Paclitaxel, Irinotecan, Folfox, Folfiri, many as a combo treatment. There is a useful article explaining the role of Ki-67 in determining optimal chemotherapy in high grade neuroendocrine tumors.
Horses for Courses
However, cytoxic chemotherapy is often inadequate for treatment of Grade 1 and 2 Neuroendocrine tumours, because these tumours tend to have a well-differentiated histology and low proliferation index – standard chemotherapy does not appear to like their slow cytokinetic growth. Although they tend to work better on certain parts of the anatomy, e.g. pancreatic NETs. Of interest is a statistic from NET Research Foundation indicating that 23% of patients who were to be prescribed chemo had their treatment changed to a non-chemo option following a Ga68 PET scan. Read more here.
For second line therapy (including for well differentiated NETs where other conventional treatments are not working), chemo may be given. These include (but not limited to) Capecitabine, Temozolomide, Bevacizumab, Xelox, Folfox. There are other specialist chemos for Mixed Neuroendocrine Non-Neuroendocrine Neoplasms (MiNEN).
Does it work on the lower grade NETs?
Capecitabine plus Temozolomide (CAPTEM for short) is fast becoming a treatment used on certain lower grade NETs. Dr Robert Fine says the results of the trial showed “tremendous responses in every neuroendocrine tumor”. The treatment elicited a response rate of 45% and a stable disease rate of 52% including those with certain types of NETs and pituitary tumours – types of neuroendocrine tumour that are notoriously ‘chemoresistant’. You can read more about this here (click here) and you can also listen to Dr Fine enthusiastically talking about this on a short You Tube video clip – (click here). Clearly it is not going to work for all.
Other CAPTEM Resources:
There’s a very interesting report on the use of CAPTEM in NETs – (click here)
In Australia, they’re also using a combo treatment of chemo (CAPTEM) and PRRT – I blogged about this click here.
There’s also a useful surgical technique which includes the use of intra-operative chemo, known as “Chinese Dumplings” – I wrote about this click here.
My Oncologist did mention Chemotherapy on my initial meeting and I was once scheduled to have a chemo-embolization (or TACE, Trans-arterial Chemo Embolization) but it never occurred due to post surgical routing issues. Clearly TACE is more targeted than conventional and generally systemic chemotherapy techniques.
Chemotherapy vs Targeted Biological Agents
I often see people describing Somatostatin Analogues (Lanreotide/Octreotide), Afinitor (Everolimus) and Sutent (Sunitinib) as chemo but that’s isn’t technically correct, and I’ve yet to find a NET Specialist or a NET Specialist Organisation who classifies these drugs as chemo. See my article “Chemo or not Chemo” (click here).
Future of Chemo?
A lot is written about how much longer chemo will be around. It gets a bad press – I suspect mainly due to the side effects. There are suggestions that it will eventually be replaced by Immunotherapy and other treatments downstream. However, immunotherapy is really still in its infancy and there remains a lack of long term data on success rates and side effects. I suspect chemo will be around for a while longer, particularly for cancers where it has a track record of curing according to ASCO. Very recently (June 2018), cancer experts said that chemo will be around for a long time yet – read more here
If in doubt about suitability for any form of chemo, patients should seek the advice of a NET specialist.
There’s a constant debate regarding the validity of the term ‘Carcinoid‘. I’ve posted about this a few times and as far as I know, the debate has been raging for some years.
You may have noticed that ‘Carcinoid’ is often used as a standalone word and tends not to be suffixed with the word ‘Cancer’ or ‘Tumour’ – unlike Bowel Cancer, Breast Cancer, Prostrate Cancer, Lung Cancer, Brain Tumour, etc. Nobody goes around saying “Breast” or “Bowel” do they? But they happily say “Carcinoid”. Unfortunately, the term ‘Carcinoid’ has become entrenched in both pathology and clinical literature over the past 100 years. The main problem with the word Carcinoid is that it means different things to different people. Some use the term almost exclusively to designate serotonin-producing tumours that arise from the enterochromaffin cells that can result in carcinoid syndrome i.e. most commonly in the appendix, small intestine, stomach, lung, rectum and uncommonly in other places. Some use it to (incorrectly) refer to all Neuroendocrine Tumours. The most worrying connotation of the use of the word ‘Carcinoid’ is the belief that they all have benign clinical and biological behaviour. That is dangerous thinking and has the potential to kill people. Fortunately, NET specialists are starting to move away from using the word – check out the quote below:
The following history of ‘Carcinoid’ is well documented: Siegfried Oberndorfer (1876-1944) became the first to adequately characterise the nature of Carcinoid tumours and refer to them as “benign carcinomas.” During his tenure at the Pathological Institute of the University of Munich, Oberndorfer noted in 1907 that the lesions were distinct clinical entities and named them “karzinoide” (“carcinoma-like“), emphasizing in particular their benign features. However, In 1929 he amended his classification to include the possibility that these small tumours could be malignant and also metastasise. (Author’s note – a name change would have been handy at this point).
100 years later
NANETS, UKINETS and ENETS seem to defer to the WHO classification nomenclature and it is here another term is introduced – Neuroendocrine Neoplasms (NENs). NANETs state that “all of the entities under discussion are neoplastic, and neoplasm is therefore a more accurate term than tumor, which means only a mass“. These organisations tend to use the term Neoplasm as a catch-all for all Neuroendocrine disease and then the term ‘tumor’ and ‘carcinoma’ applies to well and poorly differentiated respectively. It’s worth noting that since 2010, the WHO classification is based on the concept that all NETs have malignant potential, and has therefore abandoned the division into benign and malignant NETs and tumours of uncertain malignant potential. Neuroendocrine Carcinoma is malignant by defintion. All of this has been reinforced in the 2017 publication. The term ‘Carcinoid’ is conspicuously missing from these texts.
To put it simply – the term ‘carcinoid’ is no longer credible
Due to its historical meaning, Carcinoid does not adequately convey the potential for malignant behaviour that accompanies many of these neoplasms as described above. The term Carcinoid decodes to ‘Carcinoma like’. Contextually “Carcinoid Cancer” decodes to “carcinoma like cancer” which is, of course, totally misleading and its use simply perpetuates the claim by some that it is ‘not a proper cancer’. If we only needed one reason to ditch the word ‘Carcinoid’, this would be it.
I mentioned confusion above and this has led to a hybrid effect of naming the condition. For example, there is a tendency by some (including medical establishments and patient organisations) to use the term ‘Carcinoid’ and ‘Neuroendocrine Tumors’ interchangeably which is patently incorrect. Neither is it helpful that many patients and organisations continue to refer to this disease as “Carcinoid Neuroendocrine Tumor”, “Neuroendocrine Carcinoid Tumor”, “Carcinoid/NET”, “CNET”; and many other variations along these lines. Many seemingly credible organisations will say “Carcinoid and Neuroendocrine Tumors” not realising it’s a contradiction in terms. Continued use of the term in any phrase or standalone context is not doing our case for recognition any good – it’s bad enough that some seem to cling to outdated and invalid diagnostic clichés and icons from the 1980s. All of it needs to go.
I know I’m not alone in this thinking given the decrease of its use in the NET world, including NET Specialists (see lead graphic) and NET Specialist organisations (some have changed their names). There’s an interesting article written by a NET specialist where the term ‘carcinoid’ is described as “unfortunate”, “misleading”, “outmoded”, “archaic”, “confusing” and “misnomer”. Exactly! In the recent SEER NET study, a NET specialist reaffirmed this thinking by stating that “the belief these tumors did not metastasize, did not reach any great size, and appeared harmless, has since been proven false”. Continued use of the term ‘Carcinoid’ has the potential to regress this thinking. We must not let this happen.
So what terms should we be using?
People and organisations will be out of date with modern Neuroendocrine Neoplasms nomenclature and some will still want to continue with their own nomenclature (….. and because of the confusion, some will fall into both categories not realising they’re out of date). Here’s a classic example of the problem we face – the American Cancer Society(ACS) does not even list Neuroendocrine Tumor as a cancer type. Instead you can find “Gastrointestinal Carcinoid Tumors” and “Lung Carcinoid Tumor”. You’ll find Pancreatic NETs inside Pancreatic Cancer. Americans should harangue the ACS to get this right. I could go on with many similar observations on seemingly respectable sites. I intentionally used a US example as this country appears to be way behind in the changes to NET nomenclature, pretty surprising as they tend to be at the forefront of many other aspects in the world of NETs.
Personally, I think the acceptance of a common worldwide nomenclature should come from the World Health Organisation (WHO) classification for Neuroendocrine Neoplasms. They are divided into a number of chapters including ‘Endocrine Organs’, Digestive System, Lung Tumours….. and no doubt some others. Frustrating, but medical people tend to look at things in anatomical terms. Nonetheless, the agreed classification nomenclature for the whole group of Neuroendocrine Neoplasms can be found with some research and access to clinical publications. The correct nomenclature should then be flowed down in regional groupings, e.g. ENETS representing Europe, NANETS representing North America, etc. As I understand it, ENETS and UKINETS are already essentially aligned with WHO and NANETS appears to be. From these organisations, the use of the correct terminology should then rub off on patients, patient advocate organisations and general cancer sites. However, the biggest challenge will be with hospitals/medical centres, cancer registries and insurance companies whose medical record processing is run using reference data (think drop down selections and database structures). Easier said than done but ‘change’ always has to start somewhere. Technically it has started (albeit late) as the big NET medical organisations are already starting to reduce the use of outmoded words such as ‘carcinoid’.
I once argued that the term ‘carcinoid’ needed to be retained as it represented a histopathological grouping of a particular type of NET comprising mostly appendiceal, stomach (gastric), rectal, small intestine and lung NETs. However, reading through the ENETS 2016 guidance in conjunction with the most up to date WHO classification publications, I’ve changed my mind after noticing they no longer use the word ‘Carcinoid’ in relation to a tumor type. Rather, they use the latest WHO terms above and then use the anatomy to distinguish the different types of NET (like we already do for Pancreatic NET or pNET).
Perhaps patients can lead the way here ………
Rather than say:
‘Carcinoid’ or ‘Carcinoid Tumor’….. why not say Neuroendocrine Tumor or NET (adding your primary location if required – see below);
‘Carcinoid Cancer; ….. why not say Neuroendocrine Cancer;
‘Lung Carcinoid’ ….. why not say Lung NET (adding typical or atypical if required);
‘Small intestine Carcinoid’, why not say Small Intestine NET (or ‘SiNET which is becoming popular); p.s. I’m not a fan of ‘small bowel’ due to the potential for confusion with the widely used term ‘bowel cancer’);
‘Gastric Carcinoid’, why not say Gastric NET (adding your type if required);
‘Rectal Carcinoid’, why not say Rectal NET;
‘Appendiceal Carcinoid’, why not say Appendiceal NET;
…. and so on. And you can add your stage and grade/differentiation for a richer picture.
You can listen to a very well known NET Specialist say something similar in this videohere.
Worth noting that even ENETS and NANETS cannot agree on tumor type terminology – the latter uses Small Bowel NETs (SBNETs) whereas ENETS uses Small Intestine NENs (SiNENs). I did say it’s easier said than done.
As I said above, the term ‘Carcinoid’ has become entrenched in both pathology and clinical literature over the past 100 years so it will still appear in many texts and need to be searchable online to support medical and advocacy business. However, these are technical issues and I don’t therefore believe people need to use the terms to make them searchable online. I tag all my posts with ‘Carcinoid’ even if I don’t mention the word in my text. I have started only using the term for context when it is required and am currently reviewing all of my posts to ensure that is still the case.
Hang on…what about Carcinoid Syndrome
When someone wants to know which syndrome you have, you can’t just state (say) “small intestine syndrome” or “midgut syndrome”. ‘NET Syndrome’ doesn’t work either as there are several NET syndromes. This has led to the situation where people try to drop the word ‘carcinoid’ and just say “the syndrome” which is even more confusing! I accept this one is a difficult challenge but I don’t believe it’s insurmountable, just needs some willpower and agreement.
What about Carcinoid Heart Disease
Personally I don’t see why this cannot be renamed to ‘Neuroendocrine Heart Disease’ or its technical name – ‘Hedinger syndrome’.
What about Carcinoid Crisis
World renowned NET specialists already make statements that these issues can apply to all types of NET; and it’s well-known that a similar crisis situation already applies to other types e.g. Pheochromocytomas.I cannot see why something along the lines of ‘Neuroendocrine Crisis’ or ‘NET Crisis’ would not be acceptable.
We as patients are unlikely to be able to force changes on the medical and insurance communities but we can be a ‘force for change’ by setting the example of using a correct and more apt terminology to describe our disease.
Thanks for listening
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After diagnosis in July 2010, holidays were put on the back burner, there were too many problems and too many risks – not least of which was the lack of overseas insurance cover for my condition (well, I’m sure they’d quote me but could I afford it?). After 2 years of treatment including several surgeries, I was feeling more confident and my body had become stronger, holidays were put back on the agenda, but nothing too strenuous, nothing too far away.
However, 2 more years down the road, Chris and I are just back from a 12,000 mile round trip (21 hours on an aeroplane), around 1200 miles/20 hours of driving from beaches to deserts and mountains and back again, 8 different hotels and some great sights and adventures including 200 miles of driving in the Californian ‘wilderness’ picking up some sections of Route 66. The picture in the blog needed a ‘white knuckle’ cable car ride up to 8500 feet followed by a 2 hour hike in noticeably thinner air. Worth it!
Did I have issues? Yes. Were they inhibiting? Not really. Was I exhausted on return? Yes (….I still am!). Did my travel insurance cover me for NET Cancer treatment? No. Was it worth it? Absolutely, I was extremely confident I wouldn’t have a NET Cancer problem and was therefore happy to take the risk (everyone needs to take their own decisions). My travel insurance at the time covered me for all other medical emergencies worldwide and within the European Union (EU) countries I’m covered for medical treatment using the E111 system – however, the latest trip was to California where medical treatment can be very expensive for the uninsured. Note – in 2016, I’m now covered for NET treatment worldwide.
Holidays now have to be planned around treatment, mainly monthly injections – not too much of a drama. I have to take my daily blood thinning injections with me on the plane. I have a letter from a Doctor to explain but I’ve always been waved through without question. When required, I will change a monthly injection date by up to a week each side – no issues to date. That said, I’m impressed by the logistical talent of my friend Hilary, she’s gone to Australia for a month and has arranged an Octreotide injection whilst she’s there!
Cancer doesn’t take holidays, but I do. Sometimes, you just have to get on with it 🙂
Thanks for listening
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It’s that time again, every 6 months I need some checks. I’ve done the specialist blood test (Chromogranin A – CgA) and the 24 hour urine (5HIAA) and am waiting on my CT scan appointment. It’s also time for my annual Echocardiogram. I then see my Consultant and he delivers the news. Happy days 🙂
I positively look forward to my tests and I cannot wait to get into that scanner! ‘Scanxiety’ isn’t in my dictionary. Why? Because testing is one thing that’s going to keep me alive for as long as possible. If I don’t get regularly tested, then one day I might just ‘keel over’ because something wasn’t spotted early enough. Even in the event of ‘not so good news’, I still see that as a positive because it means the testing is working and an investigation or further testing can be put into place to find the problem – and the sooner the better. Where’s that scanner, get me in it!
One of the most common posts on NET Cancer forum sites is to express personal concerns or worries about upcoming appointments or waiting on the test results. Thinking back to my own countless appointments either for testing, treatment or for receiving results, I appear to be consistently pragmatic in my approach.
The test results will be what the test results will be. Worrying about them is not going to change them!
Bring it on!
You may enjoy my article “Living with Neuroendocrine Cancer – 7 tips for conquering fear”. Read here